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This article is part of the supplement: Advances in systemic sclerosis and related fibrotic and vascular conditions

Highly Accessed Review

Pulmonary hypertension associated with sarcoidosis

Robert P Baughman

Author affiliations

Department of Internal Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati Medical Center, Holmes Eden Ave, Cincinnati, Ohio 45267, USA

Citation and License

Arthritis Research & Therapy 2007, 9(Suppl 2):S8  doi:10.1186/ar2192

Published: 15 August 2007

Abstract

Pulmonary involvement is common in sarcoidosis, an immune-mediated inflammatory disorder that is characterized by non-caseating granulomas in tissue. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, risk developing pulmonary hypertension (World Health Organization group III pulmonary hypertension secondary to hypoxic lung disease). Increased levels of endothelin (ET)-1 in plasma and bronchoalveolar lavage of some sarcoid patients suggest that ET-1 may be driving pulmonary fibrosis and sarcoidosis-associated pulmonary hypertension. Although a relationship between raised levels of ET-1 and clinical phenotype is yet to be identified, early evidence from studies of ET-1 blockade with drugs such as bosentan is encouraging. Such therapy possibly could be combined with standard anti-inflammatory agents to improve outcome.